Panic at the bile duct: how intrahepatic cholangiocytes respond to stress and injury

HR Hrncir, F Hantelys, AD Gracz - The American Journal of Pathology, 2023 - Elsevier
In the liver, biliary epithelial cells (BECs) line intrahepatic bile ducts (IHBDs) and are
primarily responsible for modifying and transporting hepatocyte-produced bile to the …

A TGFβ-ECM-integrin signaling axis drives structural reconfiguration of the bile duct to promote polycystic liver disease

SH Waddell, Y Yao, P Olaizola, A Walker… - Science translational …, 2023 - science.org
The formation of multiple cysts in the liver occurs in a number of isolated monogenic
diseases or multisystemic syndromes, during which bile ducts develop into fluid-filled biliary …

Genetic spectrum of polycystic kidney and liver diseases and the resulting phenotypes

H Yang, CJ Sieben, RS Schauer, PC Harris - Advances in Kidney Disease …, 2023 - Elsevier
Polycystic kidney diseases are a group of monogenically inherited disorders characterized
by cyst development in the kidney with defects in primary cilia function central to …

Novel α-1, 3-Glucosyltransferase Variants and Their Broad Clinical Polycystic Liver Disease Spectrum

MM Boerrigter, RHM Te Morsche, H Venselaar… - Genes, 2023 - mdpi.com
Protein-truncating variants in α-1, 3-glucosyltransferase (ALG8) are a risk factor for a mild
cystic kidney disease phenotype. The association between these variants and liver cysts is …

Heterozygosity of ALG9 in association with autosomal dominant polycystic liver disease

MM Boerrigter, R Duijzer, RHM Te Morsche… - Genes, 2023 - mdpi.com
α-1, 2-mannosyltransferase (ALG9) germline variants are linked to autosomal dominant
polycystic kidney disease (ADPKD). Many individuals affected with ADPKD possess …

Inhibition of deubiquitinase USP28 attenuates cyst growth in autosomal dominant polycystic kidney disease

Y Ren, X Zhu, K Fu, H Zhang, W Zhao, Y Lin… - Biochemical …, 2023 - Elsevier
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited
kidney disease, which is characterized by progressive growth of multiple renal cysts in …

NOTCH signalling–a core regulator of bile duct disease?

A Martinez Lyons, L Boulter - Disease Models & …, 2023 - journals.biologists.com
The Notch signalling pathway is an evolutionarily conserved mechanism of cell–cell
communication that mediates cellular proliferation, fate determination and maintenance of …

Key factors influencing paediatric COVID-19 vaccine hesitancy: A brief overview and Decision-making Trial and Evaluation Laboratory analysis

Y Wang, X Zhang - Public Health, 2023 - Elsevier
Objectives The purpose of this study was to examine the current literature on paediatric
COVID-19 vaccine hesitancy among parents and identify key influencing factors, thus …

[HTML][HTML] Sex, Genotype, and Liver Volume Progression as Risk of Hospitalization Determinants in Autosomal Dominant Polycystic Liver Disease

R Schönauer, D Sierks, M Boerrigter, T Jawaid… - Gastroenterology, 2024 - Elsevier
Background & Aims Autosomal dominant polycystic liver disease is a rare condition with a
female preponderance, based mainly on pathogenic variants in 2 genes, PRKCSH and …

[PDF][PDF] Polycystic liver diseases: from molecular basis to development of effective treatments

E Markaide, JM Bañales, PM Rodrigues… - Revista Española de …, 2023 - online.reed.es
JMB),“Euskadi RIS3”(2019222054, 2020333010, 2022333032 to JMB). La Caixa Scientific
Foundation (HR17-00601 to JMB).“Fundación Científica de la Asociación Española Contra …